N-acetylcysteine delays the infiltration of inflammatory cells into the lungs of paraquat-intoxicated rats.

We investigated a possible role for N-acetylcysteine (NAC), a well-known antioxidant and free radical scavenger, against oxidative lung damage as observed in the in vivo model of paraquat-intoxicated rats. The administration of two ip doses of 50 mg/kg NAC to paraquat-intoxicated animals did not change the glutathione status of the lungs, as determined by the measurement of nonprotein sulfhydryl (NP-SH) groups. The administration of NAC did however suppress the paraquat-induced release of chemoattractants for neutrophils in the bronchoalveolar fluid when the lavage was carried out 12 hr after the administration of 30 mg/kg paraquat. Also, in the intoxicated NAC-treated animals, the infiltration of inflammatory cells was significantly reduced, as demonstrated by the examination of the cell composition of the bronchoalveolar lavage (BAL), 24 hr after paraquat. Phorbol myristate acetate-stimulated superoxide anion production from the AM isolated from the BAL of paraquat-intoxicated nontreated animals was lower than that of controls, whereas in the NAC-treated animals, it was close to that of the controls. The obtained results indicate that NAC has a protective effect against oxidative lung damage by delaying inflammation. It also prevents the paraquat-induced reduction of superoxide anion production by stimulated AM. In the present model, however, the NAC administration regimen did not affect the survival rate of paraquat-intoxicated rats.

Improved results in osteogenic sarcoma 1973-79 vs. 1980-86: analysis of results from a single center.

Analysis of treatment results in osteogenic sarcoma patients with classical limb primary tumors and without metastasis at diagnosis or major protocol violations showed improved prognosis with a minimum follow-up of over 5.5 years when divided by years of treatment for all event-free survivors. Twelve patients treated in 1980-86 had a 5-year disease-free survival of 67% and 9 treated in 1973-79 had a 5-year disease-free survival of 33% (P = 0.0368). The improvement appeared to reflect the increase in the intensity of the chemotherapy utilized. Definitive surgery, amputation or limb salvage did not affect the outcome. With these surgical approaches the disease-free survival was 67% and 60% respectively.

Rambam-Hasharon syndrome of psychomotor retardation, short stature, defective neutrophil motility, and Bombay phenotype.

We describe 2 Arab patients, both offspring of unrelated consanguineous matings, with unusual facial appearance, severe mental retardation, microcephaly, cortical atrophy, seizures, hypotonia, dwarfism, and recurrent infections with neutrophilia. Neutrophil motility was markedly decreased but the opsonophagocytic activity was normal. Both patients lack the red blood cell (RBC) H antigen and manifest the Bombay (hh) phenotype. Familial endocardial fibroelastosis and familial tetralogy of Fallot segregated independently in one family. The occurrence of the same syndrome in 2 unrelated families suggests that the various aspects of the disorder are the pleiotropic effects of a single mutation. Homozygosity-by-descent for a deletion involving contiguous genes may explain the findings in this syndrome. Alternatively, a mutation which involves an ubiquitous GDP fucose donor rather than the enzyme (alpha 2-L-fucosyltransferase) or its substrate (glcNAc) may account for the pleiotropic manifestations in this syndrome.

Ultrastructural abnormalities in endoscopically and histologically normal and involved colon in ulcerative colitis.

Twenty colonoscopies (eight complete or almost complete; 12 short) were carried out on 15 patients with ulcerative colitis with the aim of comparing the endoscopic aspects with the light and electron microscopic features in biopsies taken from multiple sites. Patients with severe attacks were examined without prior preparation (two examinations). When the attack was mild to moderate (11 examinations), or the patient was in remission (seven examinations), two saline enemas were given up to 1 h before examination. There was a favorable correlation between the endoscopic and light microscopic features in 94.7% of the biopsies (total number of biopsies, 76). The electron microscope findings greatly exceeded those observed by light microscope and indicated that the major abnormality resides within the colonic epithelial cells. Distinctive ultrastructural changes were present both in apparently uninvolved (endoscopically and histologically) parts of colon and in inactive stages of ulcerative colitis. These findings suggest that colonic mucosal involvement may be universal, persist during clinical remission, and precede the light microscopic findings. They also support the importance of maintenance therapy.

[Malignant lymphoma of the larynx].

Primary localized laryngeal lymphoma is rare. The presenting symptoms are hoarseness of long duration and increasing dyspnea. Clinical and histological diagnosis is difficult and in some cases repeated biopsies are needed for conclusive diagnosis. Radiotherapy appears to be the treatment of choice for the disease. As in addition there may be head, neck or distant localizations, there should be a complete clinical evaluation before treatment, including lymphangiography, IV pyelography, liver and spleen scans, and total body CT scan. Although in a significant number of cases laryngeal lymphoma has been cured or has remained a localized disease, it is very likely that in some cases additional treatment for the tumor at other sites may be required. Lifelong follow-up and careful periodic evaluation by experts is imperative.

Secondary hemochromatosis due to prolonged iron ingestion.

Cases of secondary hemochromatosis caused by excessive iron ingestion are very rare. In most instances there are associated factors known to cause iron overload, such as anemia, alcoholism or the presence of the hemochromatosis allele. We report a patient who developed secondary hemochromatosis, apparently due only to excessive iron ingestion.

Effect of sucralfate on experimental colitis in the rat.

The therapeutic effect of sucralfate on ulcerated gastric and duodenal mucosa is well known. There is, however, almost no information about its activity in colitis. Experimental colitis was produced in rats by rectal instillation of 1 ml of 10 percent acetic acid, and 1.5 ml of a 20 percent suspension of sucralfate was then administered every 12 hours for various lengths of time. Study animals and appropriate controls were killed after 3, 7, 10, or 14 days. The distal colons were studied macroscopically and histologically. Colonic prostaglandin E2 levels were measured in animals killed after 3, 7, 10, or 14 days. The macroscopic score was significantly improved 10 and 14 days after induction of colitis, although the histologic appearance was unchanged. Acetic acid administration increased and sucralfate treatment reduced prostaglandin E2 levels in colitic animals on days 3 and 7, but not later. The present study supports a role for sucralfate in the treatment of colitis, but further studies on the mechanism of its effect and on its clinical activity are indicated.

Barrett's mucosa of distal esophagus with concomitant isolated Crohn's disease and intramucosal adenocarcinoma. Report of a case and analysis of the literature.

The presence in the esophagus of three distinct entities--Barrett's mucosa, Crohn's disease, and adenocarcinoma--is a very rare finding. In a 60-year-old man with a long history of heartburn and recently developed dysphagia, narrowing of the distal esophagus was found to be related to the presence of Barrett's mucosa. A short time later repeated endoscopy revealed adenocarcinoma in this area. The patient underwent esophagogastrectomy and died a few days after surgery. Findings in the surgical specimen and upon autopsy were consistent with isolated Crohn's disease of the distal esophagus as well as with intramucosal adenocarcinoma. Analysis of the data available in the literature reveals that Crohn's disease of the esophagus, although rare, clearly possesses some definite characteristics of its own. It is suggested that the presence of these three features in a single patient constitutes no more than a chance coexistence.

Anaplastic thyroid carcinoma. A clinical, histologic, and immunohistochemical study.

Twenty-six cases of anaplastic thyroid tumor were investigated and reclassified using immunoperoxidase techniques. Sections of the neoplasms were stained immunohistologically for the following thyroid associated antigens: (1) thyroglobulin, which shows a positive reaction with follicular cells of the thyroid; (2) calcitonin, which is positive in medullary carcinoma of the thyroid; and (3) leucocyte common antigen (LC), which identifies lymphomata and Factor VIII-related antigen for hemangioendothelioma. Using these methods, five cases were reclassified. Three cases were identified as lymphomata, one case was reclassified as medullary carcinoma of the thyroid, and one case was identified as hemangioendothelioma. Eleven cases were confirmed to be anaplastic carcinoma of the thyroid and ten cases were negative for all the antigens tested. There was a significant difference in the survival of the groups of patients mentioned above. Prognostic data support the suggestion that immunohistochemical methods should be used for the precise classification of anaplastic carcinoma. In this way, tumors such as malignant lymphoma and medullary carcinoma, which resemble anaplastic carcinoma histologically but have a better prognosis, can be identified. This is important for planning surgical procedures and choosing chemotherapy and/or radiotherapy.

Massive hemoptysis complicating Behçet's syndrome: the importance of early pulmonary angiography and operation.

A 16-year-old patient with Behçet's syndrome had massive hemoptysis due to a ruptured aneurysm of a segmental artery of the left lung. Emergency left lower lobectomy was performed. The patient is well 12 months after operation. There have been no further episodes of hemoptysis.

Carcinoma of the prostate metastatic to the maxillary antrum.

Metastatic carcinoma of the maxillary antrum is an extreme rarity. Until 1980, less than 100 cases with distant primaries metastatic to the entire sinonasal tract had been reported. In a review of these cases, we found no mention of primary prostate cancer metastatic to the antrum. The purpose of this paper is to document the first case of this entity.

Malignant melanomas of female urethra.

Primary melanoma of the female urethra is extremely rare. Treatment by surgery, radiation, and chemotherapy has been uniformly ineffective. We herein report on 2 patients with primary malignant melanoma of the urethra treated with immunotherapy and chemotherapy. The first patient died four years after the initial diagnosis, and the second died after two years. Chemo-immunotherapy may be considered as an additional, palliative form of treatment in the management of primary melanoma of the female urethra, but cystourethrectomy should be the initial form of treatment.